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Perivascular epithelioid cell tumors (PEComa)s are mesenchymal neoplasms located at various anatomic sites, which usually express both melanocytic and myogenic markers. A 60-year-old woman underwent laparotomy for a huge, heterogeneous, right ovarian mass. The histological examination of the surgical specimen revealed a neoplasm consisting of both cells with clear or eosinophilic cytoplasm and spindle cells in a myxoid stroma. Immunostaining was positive for human melanoma black-45, h-caldesmon, desmin, actin, and transcription factor 3. Cell atypias were moderate, mitoses were 4/10 high power fields (HPF) and margins were focally infiltrative. These findings pointed to a diagnosis of ovarian PEComa. Twenty-five months later, two subcutaneous lesions were surgically removed on the left trapezius muscle and the median subumbilical area, respectively. The former was a desmoid fibromatosis, whereas the latter was a recurrence of PEComa with greater nuclear pleomorphism and higher number of mitoses (26/50 HPF) compared to the primary tumor. The patient was free of disease 11 months later. A long-term follow-up of gynecological PEComas is strongly recommended. Copyright © 2021 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.


Angiolo Gadducci, Clara Ugolini, Stefania Cosio, Fabio Vistoli, Emanuele Federico Kauffmann, Ugo Boggi. Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Ovary: A Case Report and Review of the Literature. Anticancer research. 2021 Sep;41(9):4483-4488

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PMID: 34475073

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